Early diagnosis of diseases is key not just for better treatment outcomes but also for better quality of life, more so if it involves children.
And this is what is crucial for children born with extra hepatic biliary atresia, a congenital condition in which the bile duct connecting the liver and intestine is absent.
Doctors say global statistics show that one in 10,000 children is born with this defect, the cause for which is unknown.
“It is a development malformation as the tube fails to form. The bile is produced in the liver and its main function is absorption of fat. In the absence of the duct, it stays in the liver and goes on to damage it. It presents as jaundice in the newborn,” said Naresh P. Shanmugam, consultant, paediatric hepatologist, Global Health City.
The bile gives the yellow colour to stool, and in its absence, the stool is white or pale in colour, he said. “Diagnosis is difficult as many babies suffer from jaundice at birth but if it persists, the colour of the stool changes and this is one of the main indicators but is often missed by parents,” he said.
Early surgery is crucial to avoid complications, said doctors, adding Yellow Alert, which is a chart consisting of all stool colours, could be circulated among paediatricians to help in diagnosis.
Priya Ramachandran, consultant paediatric surgeon based in Kanchi Kamakoti CHILDS Trust Hospital, said the success rate of surgery is 80 per cent if done within 30 days of birth, and 30 to 40 per cent if done after 90 days.
“Surgery involves connecting the intestine directly to the liver so the bile drains into it,” she said.
Having performed the surgery for 15 children last year, she said, “If the surgery is not successful, then the child might require liver transplant in the infancy stage itself.”
The surgery should be performed by surgeons who carry out at least five such procedures a year, so the results are good, said Mohamed Rela, director, Institute of Liver Diseases and Transplantation, Global Hospitals Group.
“It determines if the patients can survive without liver transplant or if they should come for early transplant. Hence, there is need for expertise. We need awareness among paediatricians on picking up the symptoms early,” he said.
This condition is fairly common and in the long run, 60 to 70 per cent of children might require liver transplant, said Dr. Ramachandran.
“The transplant and immunosuppressant drugs involve huge costs and hence, the State government could help,” she said.
